Early autophagic response in a novel knock-in model of Huntington disease.

  title={Early autophagic response in a novel knock-in model of Huntington disease.},
  author={Mary Y Heng and Duy K. Duong and Roger L Albin and Sara J Tallaksen-Greene and Jesse Hunter and Mathieu J Lesort and Alex Osmand and Henry L. Paulson and Peter J Detloff},
  journal={Human molecular genetics},
  volume={19 19},
The aggregation of mutant polyglutamine (polyQ) proteins has sparked interest in the role of protein quality-control pathways in Huntington's disease (HD) and related polyQ disorders. Employing a novel knock-in HD mouse model, we provide in vivo evidence of early, sustained alterations of autophagy in response to mutant huntingtin (mhtt). The HdhQ200 knock-in model, derived from the selective breeding of HdhQ150 knock-in mice, manifests an accelerated and more robust phenotype than the parent… CONTINUE READING


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